Flynn J T, Schulman S L, deChadarevian J P, Dunn S P, Kaiser B A, Polinsky M S, Baluarte H J
Department of Pediatrics, St. Christopher's Hospital for Children, Temple University School of Medicine, Philadelphia, Pennsylvania 19134-1095.
Pediatr Nephrol. 1992 Nov;6(6):553-5. doi: 10.1007/BF00866503.
A child with congenital nephrotic syndrome underwent renal transplantation, was treated for acute rejection, and then developed nephrotic syndrome and renal failure. He was felt to have minimal change disease on allograft biopsy, but failed to respond to therapy with corticosteroids. Cyclophosphamide was substituted for cyclosporine and rapidly induced a complete remission of his nephrotic syndrome. We feel that this case not only represents an important example of a useful therapeutic approach to the child with congenital nephrotic syndrome who develops nephrotic syndrome post transplantation, and also raises questions concerning the pathogenesis of congenital nephrotic syndrome.
一名患有先天性肾病综合征的儿童接受了肾移植，因急性排斥反应接受治疗，随后出现肾病综合征和肾衰竭。移植肾活检显示为微小病变病，但对皮质类固醇治疗无反应。环磷酰胺替代环孢素后，迅速使他的肾病综合征完全缓解。我们认为，该病例不仅是对移植后发生肾病综合征的先天性肾病综合征患儿有用治疗方法的一个重要实例，也引发了关于先天性肾病综合征发病机制的问题。
